Mooren Ulcer

7/29/2019 Mooren Ulcer

1/14

Mooren's ulcer : Current concepts in management

VS Sangwan, P Zafirakis, CS FosterMassachusetts Eye and Ear Infirmary, Department of Ophthalmology, Boston, USA

Correspondence Address:V S Sangwan

Massachusetts Eye and Ear Infirmary, Department of Ophthalmology, BostonUSA

Source of Support: None, Conflict of Interest: None

Abstract

Mooren's ulcer is strictly a peripheral ulcerative keratitis (PUK) with no associated

scleritis. It occurs completely in absence of any diagnosable systemic disorder that could

be responsible for the progressive destruction of the cornea. The aetiology of Mooren'sulcer remains uncertain. However, recent studies indicate that it is an autoimmune disease

directed against a specific target molecule in the corneal stroma, probably triggered in

genetically susceptible individuals by one of several possible provocateurs. Advanceshave been made in the management of this disease. Immunosuppressive therapy has been

shown increasingly successful in patients unresponsive to conventional treatment and in

bilateral progressive destructive ocular disease.

Keywords: Moorens ulcer, Peripheral ulcerative keratitis (PUK)

How to cite this article:

Sangwan VS, Zafirakis P, Foster CS. Mooren's ulcer : Current concepts in management.

Indian J Ophthalmol 1997;45:7-17

How to cite this URL:

Sangwan VS, Zafirakis P, Foster CS. Mooren's ulcer : Current concepts in management.Indian J Ophthalmol [serial online] 1997 [cited 2009 Jul 15];45:7-17. Available

from: http://www.ijo.in/text.asp?1997/45/1/7/15030

Mooren's ulcer was first described by Bowman in 1849[1], and then by McKenzie in

1854 as "chronic serpiginous ulcer of the cornea orulcus roden"[2]. Mooren's name,however, became attached to this rare disorder because of his publication of cases in
http://www.ijo.in/text.asp?1997/45/1/7/15030http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref1http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref2http://www.copyright.com/ccc/openurl.do?sid=Medknow&issn=0301-4738&servicename=all&WT.mc_id=Medknowhttp://www.ijo.in/login.asp?rd=article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwanhttp://www.ijo.in/text.asp?1997/45/1/7/15030http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref1http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref2


2/14

1863 and 1867[3]. He was the first to clearly describe this insidious corneal problem and

define it as a clinical entity. In 1902, Nettleship summarized the accumulated reported

experience with the disorder in a classic article wherein he reviewed 67 previouslypublished cases and added 11 new cases from his experience[4]. Mooren's ulcer is a

painful, relentless, chronic ulcerative keratitis that begins peripherally and progresses

circumferentially and centrally. Mooren's ulcer is, by definition, idiopathic occurring incomplete absence of any diagnosable systemic disorder that could be responsible for the

progressive destruction of the cornea. It also is strictly a peripheral ulcerative keratitis

(PUK), with no associated scleritis. Absence of scleritis is of substantial importance,since many of the misdiagnosed cases had the peripheral ulcerative keratits (PUK) in

association with adjacent scleritis, necrotizing or otherwise. Its exact pathophysiology

remains uncertain, although a growing body of evidence indicates that it is an

autoimmune disease directed against a specific target molecule in the corneal stroma,probably triggered in the genetically susceptible individuals by one of several possible

provocateurs. Despite the advances made in management of this disorder, a significant

percentage of cases remain refractory to available therapies and end in severe visual

morbidity. There are 287 cases described in world ophthalmic literature[5] ; some of thecases included in reports of "Mooren's ulcer" are in fact not true Mooren's ulcer, but

rather instances of individuals with PUK as the presenting manifestation of an occultsystemic disease.

Epidemiology

Mooren's ulcer is a rare disorder, typically seen in healthy adult men[6] with no evidence

of systemic disease. It has been documented in a 3-year old child.[7] Wood and Kaufman

having reported 9 cases, concluded that there were two clinical types of Mooren's ulcer.[8] The first, limited type, is usually unilateral, with mild to moderate symptoms,

generally responds well to medical and surgical treatment. This type is believed to occur

in older patients and has became known as typical or benign Mooren's ulcer. In contrast,

the second type is bilateral, with relatively more pain and generally a poor response totherapy. The bilateral variety, once believed to occur primarily in younger patients,

became known as atypical or malignant Mooren's ulcer. The benign type is quater time

bilateral and the malignant type 75% time bilateral[5],[9]. Usually bilateral progressiveform of this disease occurs in older individuals. Keitzman[10] published a series of 37

cases of progressive Mooren's ulcer in Nigeria affecting primarily healthy men between

the age of 20 and 30 years and the clinical course was very rapid, with total involvementand destruction of the cornea within 6 weeks. Perforation occurred in 36% of the patients.

A later report confirmed that the malignant form was the more common form occurring

in the young Nigerian men.[11] As a result, the generalized belief has developed that theprogressive and relentless atypical form of Mooren's ulcer has a predilection for young,

black men. Lewallen and Courtright[5], in their review of the published series of

Mooren's ulcer, suggest that many of our current concepts of epidemiology of Mooren's

ulcer are not supported by available data. They found that younger patients had bilateraldisease less frequently than older patients (1.5:1) regardless of race and Caucasians were

more than twice as likely to have bilateral disease than blacks. Although they found that
http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref3http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref4http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref5http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref6http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref7http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref8http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref5http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref9http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref9http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref10http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref11http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref5http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#tophttp://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref3http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref4http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref5http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref6http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref7http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref8http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref5http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref9http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref10http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref11http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref5


3/14

men were 1.6 times more likely to have Mooren's ulcer than are women, it was pointed

out that it could be due to factors such as increased incidence of ocular trauma in men (an

association with Mooren's ulcer is reported by some) or cultural patterns that discouragefemale clinic attendance in certain countries.

Lewallen and Courtright[5] assert that the available data are flawed by a collection periodof more than 85 years, differences in the basic criteria for definition of the disease, poor

documentation and follow-up, and problems inherent in non-population-based data

collection done in 20 series from more than 14 different countries. It is concluded that anaccurate epidemiologic understanding of Mooren's ulcer must await a standardized case

definition, uniform documentation and follow-up of afflicted patients, and population-

based data.

Aetiology

Different entities have been associated with Mooren's ulcer, often leading to conjecturethat there may be a causal relationship. An association with helminthiasis has been

suggested in Nigeria.[11] Schanzlin[12] speculated that the antigen-antibody reaction to

helminth toxins deposited in the peripheral cornea provoked the inflammation and

ulceration. However, the association of Mooren's ulcer is not proved even in endemicareas of ascariasis. Recently in 2 patients with bilateral Mooren's ulcers, chronic hepatitis

C infection was documented.[13],[14] and they improved after treatment of the hepatitis

with interferon 2b. The authors proposed that molecular mimicry may be involved, withthe hepatitis C virus stimulating an autoimmune response to corneal antigens through

cross-reacting epitopes. Alternatively, they also proposed that deposition of immune

complexes in limbal or peripheral corneal tissues may lead to an immune response andrelease of proteolytic enzymes. Again, chronic hepatitis C infection is not a rare disorder

and if a causal relationship is present, Mooren's ulceration would be expected to be seen

more frequently. Other infections that have been associated with Mooren's ulcer include

herpes simplex[15]and zoster[16], syphilis and tuberculosis.[17] Mooren's ulcer has alsobeen reported following local corneal diseases. Specific associations have included

physical trauma[9], foreign bodies[17], chemical burns[18]and surgical procedures such

as cataract extraction, penetrating keratoplasty[19],[20] and lamellar keratoplasty.[21]Some of these may not represent true cases of strictly defined Mooren's ulcer and even

for those that do, definitive associations and causal relationships have not been

demonstrated.

Pathophysiology

The precise pathophysiological mechanism of Mooren's ulceration remains unknown, butthere is much evidence to suggest that it is an autoimmune process, with both cell-

mediated and humoral components. On pathological examination plasma cells,

neutrophils, mast cells and eosinophils have been found in the involved areas.[22][23]
http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref5http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref11http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref12http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref13http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref14http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref15http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref15http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref16http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref16http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref17http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref9http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref9http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref17http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref17http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref18http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref18http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref19http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref20http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref21http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref22http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref22http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref23http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#tophttp://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#tophttp://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref5http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref11http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref12http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref13http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref14http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref15http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref16http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref17http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref9http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref17http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref18http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref19http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref20http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref21http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref22http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref23


4/14

[24][25] Brown[22] has demonstrated high levels of proteolytic enzymes in the affected

conjunctiva. Foster and colleagues[23] found numerous activated neutrophils in the

involved areas and proposed that these neutrophils are the source of the proteases andcollagenases that degrade the corneal stroma. They were able to stimulate the

lymphocytes of a Mooren's patient to blastogenic transformation in response to normal

corneal stroma. Additional evidence for cell-mediated autoimmune phenomena includedemonstration of a positive macrophage migration inhibition response to corneal antigens

presented to lymphocytes from Mooren's ulcer patients[26]. Furthermore, Murray and

Rahi[24] noted that, systemically, there is a decrease in the number of suppressor T cellsrelative to the number of helper T cells in Mooren's ulcer patients. From this it was

proposed that unregulated helper T cells could induce production of autoantibodies,

resulting in the deposition of immune complexes, complement activation, inflammatory

cell infiltration, and proteolytic enzyme release. Schaap and associates[27] used indirectimmunofluorescent techniques and demonstrated circulating IgG antibodies to human

corneal and conjunctival epithelium in patients with Mooren's ulcer. In addition,

antibodies and complement have been demonstrated bound to the conjunctival epithelium

in the affected areas.[25],[28]Elevated serum IgA levels[26]and circulating immunecomplexes in patients with Mooren's ulcer have also been reported.[29] Martin and

colleagues[30] have proposed a mechanism for the perpetuation of the ulcerative process,suggesting that a systemic disease, infection, or trauma may alter corneal antigens,

stimulating both humoral and cellular responses. In the process, complement activation

leads to neutrophil chemotaxis and degranulation with release of collagenases, causing

corneal melting and further alteration and exposure of altered corneal antigens, thusperpetuating the process. This cycle continues until the entire cornea is consumed.

Gottsch and associates[20] in immunologic studies of a patient with Mooren's ulcer

demonstrated cellular and humoral immune responses to bovine corneal antigens. Serumfrom this patient was used to purify a cornea-associated antigen (Co-Ag) from bovine

corneal stromal extracts.[31] The amino acid sequence of Co-Ag has been studied.[32]

The protein contains 70 amino acids in a single chain and lacks cysteine, tryptophan, andmethionine residues. The results of this study suggest that Co-Ag is a new member of the

Ca2+-binding protein of the S-100 family of proteins and could provide an important

framework to search for sequence similarity with microbial proteins as possible substratefor molecular mimicry and for identification of possible pathogenic epitopes in Co-Ag.

Despite the accumulating evidence that cell-mediated and humoral immune mechanisms

are present in Mooren's ulcer, it is not known with certainty that they are involved

directly in the pathogenesis of the disease. It remains possible that they simplyaccompany the corneal destruction that is caused by another mechanism.

Pathology

The histopathology of Mooren's ulcer suggests an immune process. Young and

Watson[33] studied the corneas of three patients who underwent grafting. They observed

that the involved limbal cornea consisted of three zones. The superficial stroma wasvascularized and infiltrated with plasma cells and lymphocytes. In this region, there was

destruction of the collagen matrix. Epithelium and Bowman's layer were absent. The
http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref24http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref25http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref22http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref23http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref26http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref24http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref27http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref25http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref25http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref28http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref28http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref26http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref26http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref29http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref30http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref20http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref31http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref31http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref32http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref33http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#tophttp://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref24http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref25http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref22http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref23http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref26http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref24http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref27http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref25http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref28http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref26http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref29http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref30http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref20http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref31http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref32http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref33


5/14

midstroma showed hyperactivity of fibroblasts with disorganization of the collagen

lamellae. The deep stroma was essentially intact but contained a heavy macrophage

infiltrate. Descemet's membrane and the endothelium were spared. Heavy neutrophilinfiltration, as well as dissolution of the superficial stroma, were present at the leading

edge of the ulcer.[22],[33]These neutrophils showed evidence of degranulation. The

adjacent conjunctiva shows epithelial hyperplasia and a subconjunctival lymphocytic andplasma cell infiltration.[22] Frank vasculitis is not present,[23] and numerous eosinophils

may present in the nearby involved conjunctiva[8] during the course of healing.

Clinical features

Patients with Mooren's ulcer usually complain of redness, tearing, and photophobia, but

pain is typically the outstanding feature. The pain often is incapacitating and may well beout of proportion to the inflammation. There may also be a complaint of decreased visual

acuity, which may be secondary to associated iritis, central corneal involvement, or

irregular astigmatism due to peripheral corneal thinning. On examination, the diseasemay be noted to begin with several patchy, peripheral stromal infiltrates that then

coalesce, more often in the medial and lateral quadrants than in the superior and inferior

ones. An epithelial defect and a shallow furrow then develop in this area [Figure:1].

Generally, there is involvement of the limbus, in contrast to some other forms of PUK,

such as that seen with rheumatoid arthritis or staphylococcal marginal disease.[9],[34]

The ulcerative process first spreads circumferentially and then centrally to involve theentire cornea eventually. The anterior one-third to one-half of the stroma is involved,

characteristically with a steep, overhanging edge [Figure:2]. Healing and vascularization

then follow, with the disease slowly running its course over 4 to 18 months.

Portions of the ulcer may be quiescent while others are active. The end- stage result is

typically a scarred, vascularized cornea that may be thinned to less than half of its

original thickness. As the end stage of the process approaches, the patient may experiencesudden relief from the excruciating pain that has been present throughout the course of

the disease.

Iritis sometimes is associated with Mooren's ulcer. Hypopyon is rare unless secondary

infection is present. Glaucoma and cataract may complicate the process. Perforation has

been noted in up to 36% cases, often associated with minor trauma to the weakenedcornea.[10]

Diagnosis

As we have stated, Mooren's ulcer is idiopathic, and it must be reemphasized that the

characteristic features reviewed must occur in absence of any systemic process that may

cause PUK. Thus, it is a diagnosis of exclusion. The differential diagnosis is that of
http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref22http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref22http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref33http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref33http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref22http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref23http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref8http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref9http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref9http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref34http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref10http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#tophttp://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#tophttp://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref22http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref33http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref22http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref23http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref8http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref9http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref34http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref10


6/14

peripheral ulcerative keratitis and is extensive (Table).

Infectious aetiologies should be excluded by appropriate cultures, because microbialkeratitis can rapidly progress and are usually amenable to antibiotic therapy. Mooren's

ulcer is easily distinguished from the noninflammatory corneal degenerations, such as

Terrien's or Pellucid marginal degeneration, in which the epithelium remains intact andpain is absent. The presence of Mooren's-like ulcer requires an extensive search for occult

and potentially lethal systemic diseases. A thorough medical history and examination is

mandatory, as is comprehensive laboratory investigation. This investigation may includea complete blood count with evaluation of the differential count, platelet count,

erythrocyte sedimentation rate, rheumatoid factor, complement fixation, antinuclear

antibodies (ANA), antineutrophil cytoplasmic antibody (ANCA), circulating immune

complexes, liver function tests, VDRL and fluorescent treponemal antibody absorption(FTA-ABS) tests, blood urea nitrogen and creatinine, serum protein electrophoresis,

urinalysis, and a chest roentgenogram. Additional testing is done as indicated by the

review of systems and physical examination.

DIFFERENTIAL DIAGNOSIS

Rheumatoid Arthritis

Rheumatoid arthritis is a frequent cause of PUK[35]. The clinical appearance of the

peripheral ulceration is not unique in rheumatoid arthritis. Frequently, there may be anassociated scleritis, a feature not characteristic of typical Mooren's ulcer. Other associated

ophthalmic findings include keratoconjuctivitis sicca (the most common ophthalmic

finding), episcleritis, and sclerosing keratitis. Advanced systemic involvement is usuallyapparent at time of ocular involvement.[35],[36]The patient's clinical profile and positive

serologic studies, in particular rheumatoid factor, will help establish the appropriate

diagnosis. Low titers, however, may be found in 1 to 5% of normal subjects, usually

elderly, in a variety of other rheumatic conditions and chronic inflammatory diseases.

Wegener's Granulomatosis

Wegener's granulomatosis is a rare multisystem granulomatous necrotizing vasculitis

with upper and lower respiratory tract and renal involvement. Ocular involvement may be

seen in up to 58% of patients, including proptosis due to orbital involvement, scleritiswith or without PUK, PUK alone, uveitis, and vasculitis.[36],[37] Orbital involvementand scleritis are the most common ophthalmic manifestations. However, PUK can be an

initial clinical manifestation and the presenting or only sign of the disease.[36],[37]

Prompt diagnosis is imperative because the initiation of immunosuppressive therapy,such as cyclophasphamide, can be both sight and life saving.[36],[37] A careful review of

systems may disclose a history of upper or lower respiratory signs or symptoms

(epistaxis, sinusitis, rhinorrhea, hoarseness, dysphagia, cough, or pleurisy), a history of
http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref35http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref35http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref35http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref36http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref36http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref36http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref37http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref36http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref36http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref37http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref36http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref36http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref37http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref37http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#tophttp://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#tophttp://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref35http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref35http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref36http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref36http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref37http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref36http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref37http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref36http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref37


7/14

microscopic hematuria, arthralagias, or skin lesions as the result of the underlying

vasculitis process. The work-up should include a haematologic and serologic survey,

including serum ANCA analysis. ANCAs are serum antibodies directed againstcomponents of primary granules of normal monocytes and neutrophils. ANCA testing has

been demonstrated to be an extremely sensitive, specific marker for systemic Wegener's

granulomatosis or a closely related group of vasculitides.[38] In general, ANCA titerstend to parallel the extent and severity of Wegener's granulomatosis and fall with the

remission of disease. Although ANCA testing has been reported to be less sensitive for

limited Wegener's granulomatosis[39], our experience with patients presenting withscleritis has found it to be exquisitely sensitive and specific.[40]

Polyarteritis Nodosa

Classic polyarteritis nodosa (PAN) is a rare multisystem disease characterized by

necrotizing vasculitis of small and medium-sized muscular arteries. The systemic

manifestations may be protean, with various constitutional symptoms. Multiple organsmay be involved, including kidney, skin, bone marrow, central nervous system, lungs,

heart, gastrointestinal and genital tract.[41] The aetiology is unknown, and the diagnosis

rests on the histopathologic identification of typical vascular changes. Visceral

angiography is helpful in establishing the diagnosis of PAN when accessible tissue is notavailable for biopsy. Microaneurysms at the bifurcation of the medium and small arteries

is highly characteristic of the disease, especially in the kidney and mesentery. Choroidal

vasculitis is the most common ophthalmic manifestation. Other ophthalmic findingsinclude PUK, conjunctival lesions, scleritis, choroiditis, retinal vasculitis, optic atrophy,

papilledema, exudative retinal detachment, central artery occlusion. The clinical

characteristics of the PUK associated with PAN are similar to Mooren's ulcer.[41]Serologic tests for hepatitis B surface antigen (HBsAg) should be obtained because

approximately 50% of patients with PAN are seropositve. Therapy with local medical

and surgical strategies may temporarily retard progression of the ulcer until control with

systemic prednisone and cyclophosphamide is achieved.

Other Collagen Vascular Diseases

Peripheral ulcerative keratitis may rarely be seen in other collagen vascular disorders

such as systemic lupus erythematosus (SLE), progressive systemic sclerosis, and

relapsing polychondritis. Diagnosis is based on a combination of clinical and laboratorycriteria.[42] Antibodies to nuclear antigen (ANA) are found in most patients with SLEbut are not necessary for diagnosis when other pertinent laboratory tests are positive and

classic features of the disease are present. ANA may be found in a wide variety of other

conditions, including old age, drug therapy with particular compounds, infections such assubacute bacterial endocarditis, certain liver diseases, rheumatoid arthritis and other

autoimmune diseases. However, antibodies to double-stranded DNA (ds DNA) are nearly

unique to patients with SLE, although antibodies to single-stranded DNA may be found
http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref38http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref39http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref40http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref41http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref41http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref41http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref42http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#tophttp://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#tophttp://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref38http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref39http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref40http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref41http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref41http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref42


8/14

in other rheumatic conditions.

No laboratory tests are specific for relapsing polychondritis. The diagnosis is based onthe clinical feature of the disease. Because the disease is episodic and tends to involve

several different organ systems (ear, nose and tracheal cartilage, joints, and the eye),

clinical criteria for the diagnosis have been established.[43],[44]

Systemic sclerosis is a generalized disorder of connective tissue characterized by

degenerative and inflammatory changes that subsequently lead to fibrosis. The skin,blood vessels, oesophagus, and synovium along with certain internal organs are affected.

The most frequent ocular finding is keratoconjunctivitis sicca, which may be seen in as

many as 70% of patients.[45] Peripheral keratitis unrelated to dry eye may be seen.

Specific antinuclear antibodies have been identified in scleroderma.[45]

Inflammatory Bowel Disease

The association between ocular inflammation and inflammatory bowel disease is well

established. Anterior uveitis, episcleritis, and scleritis are well known anterior segment

ocular manifestations. Ocular manifestations may precede or follow the onset of the

inflammatory bowel disease. There are case reports describing peripheral cornealinfiltrates at times associated with corneal ulceration.[46],[47] We have not encountered

cases with corneal ulceration.

Giant Cell Arteritis

Giant cell arteritis is an inflammatory disorder of large and medium-sized arteries

occurring mainly in older people and it characteristically involves the temporal arteries.Ocular complications are due to the ischemia caused by the involvement of the

ophthalmic artery or its branches. One case report documents peripheral corneal

ulceration as the presenting sign of temporal arteritis.[48]

Staphylococcal Marginal Keratitis

Staphylococcal marginal disease can present as a peripheral corneal infiltrate withoverlying epithelial breakdown. There is a clear intervening zone between the infiltrate

and limbus, and the keratitis is frequently accompanied by blepharitis. Patients complain

of photophobia and irritation, but the severe and debilitating pain noted with Mooren'sulcer is not described. Rapid clinical improvement can usually be seen with mild topical

steroids. The ulcers are believed to represent immune complex-mediated reactions to

microbial antigens.[49]
http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref43http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref44http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref45http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref45http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref46http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref46http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref47http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref47http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref48http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref49http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#tophttp://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#tophttp://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#tophttp://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref43http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref44http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref45http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref45http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref46http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref47http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref48http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref49


9/14

Local infectious Causes

Although microbial keratitis most commonly occurs centrally or paracentrally, infectious

causes should always be excluded in a peripheral ulcer by appropriate clinical history,

examination, and corneal cultures.

Herpes simplex may occasionally cause limbal ulceration.[50] A history of previous

herpetic disease and, occasionally a dendrite appearance to the edge of the ulcer willsuggest the correct diagnosis. The herpetic process usually begins with epithelial

ulceration and is followed by stromal infiltration. Corneal sensation may be markedly

decreased. Although acanthamoeba keratitis is relatively uncommon, it is a potentially

devastating corneal infection. It is frequently reported in young, healthy,immunocompetent patients who wear contact lenses.[51]Contaminated contact lens

solutions are frequently found. The patient typically has severe pain and may present with

a recurrent erosion, psudodendrite, or a ring, and nummular and/or neural infiltrate. The

diagnosis can be made from corneal scrapings but frequently requires a corneal biopsy.

Terrien's Degeneration

Terrien's degeneration is an important cause of peripheral corneal thinning. It differs from

Mooren's ulcer in that it is typically painless, does not ulcerate and is usually

noninflammatory. It has been reported more frequently in men and may occur at any age.The disease is usually bilateral but may be asymmetrical.[52]Terrien's degeneration

usually begins in the superior cornea, in contrast to Mooren's ulcer, which typically

begins in the interpalpebral region as a fine, punctate, stromal opacity. A clear zone existsbetween the infiltrate and the limbus, which becomes superficially vascularized. Slowly

progressive thinning follows. The thin area has a sloping peripheral border and a sharp

central edge that is highlighted by a white lipid line. The epithelium remains intact,

although bulging of thin stroma causes significant astigmatism. The thinning slowlyprogresses circumferentially but rarely centrally.[53] Perforation may be seen, usually

with minor trauma.[54]Visual symptoms are due to the astigmatism created by the

ectatic cornea. An oblique pseudopterygium may be seen in about 20% of patients.[55]Rarely, a patient may have recurrent episodes of inflammation and coexistent episcleritis

or superficial scleritis.[52]

Pellucid Degeneration

Pellucid degeneration causes bilateral, inferior corneal thinning that leads to marked,

irregular, against-the-rule astigmatism. Pain and inflammation are lacking and theepithelium is intact, thus differentiating it from Mooren's ulcer.
http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref50http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref51http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref51http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref52http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref52http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref53http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref54http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref54http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref55http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref52http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#tophttp://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#tophttp://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#tophttp://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref50http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref51http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref52http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref53http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref54http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref55http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref52


10/14

Senile Furrow Degeneration

Thinning in the lucid interval between an arcus and limbus may occur in the elderly.

However, the epithelium is intact and there is no infiltrate or inflammation. The furrow is

shallow and not vascularized, with sloping central and peripheral edges. Progression isextremely slow, and has no risk of perforation.

Ocular Rosacea

Acne rosacea is a common disease characterized by persistent erythema, telangiectasias,

sebaceous gland hyperplasia, and acneform pustules in the flush areas of the face and

neck. The classic rhinophyma is also a typical feature of advanced stage of disease.Ocular involvement, reported in up to 58% of cases, ranges in severity from mild

blephroconjunctivitis to corneal neovascularization and thinning.[56] PUK may

occasionally be seen, usually in neglected cases of acne rosacea. Inspection of the flushareas of the skin for evidence of acne rosacea in conjunction with blepharoconjunctivitis

will guide one to the appropriate diagnosis. Both the cutaneous and ocular manifestations

of acne rosacea respond well to treatment with oral tetracycline.

Leukemia

Rare cases of peripheral corneal infiltrates and PUK associated with leukemia have beenreported.[57] The clinical pattern is not distinctive, and the diagnosis must be made with

peripheral blood count or bone marrow biopsy. The mechanism of injury is unknown butmay be on the basis of vascular occlusion or corneal infiltration and release of

collagenolytic enzymes by the leukemic cells.

Treatment

Mooren's ulcer is typically progressive and relentless despite all attempts at management

and therapy. Many treatments have been tried through the years. The disease is so

uncommon that randomized and masked clinical trials to study different therapeutic

regimens are impossible. Even so, because of improvement in our understanding of thenatural history and pathophysiology of this disorder, our ability to manage Mooren's ulcer

has improved over the past two decades.

Historically, the therapeutic agents tried for Mooren's ulcer include subconjunctival

dichloride of mercury[58], carbonic and nitric acid, formalin[59] and tincture of

iodine[60], thricloroacetic acid[60], subconjunctival heparin[61], and cyanide of mercury.[62] In addition, a number of procedures, such as irradiation[63], galvanocautery[64],
http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref56http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref57http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref58http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref59http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref60http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref60http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref60http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref61http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref61http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref62http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref63http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref64http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref64http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#tophttp://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#tophttp://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#tophttp://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#tophttp://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref56http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref57http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref58http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref59http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref60http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref60http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref61http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref62http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref63http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref64


11/14

paracentesis[65], vitamin B1 injections, tuberculin injections[12], and delimiting

keratotomy[66][67][68], have been attempted with little success. More recently,

Mooren's ulcer has been treated with plasma exchange[69], periosteal grafting[70], andGore-Tex patch grafting.[71] Clearly, more study and experience are needed for this and

other potential therapies.

(For topical Cyclosporin, see below).

Today, most experts agree on a step-wise approach to the management of Mooren's ulcer,which is outlined as follows:

1. 1. Topical steroids

2. 2. Conjunctival resection

3. 3. Systemic immunosuppressives

4. 4. Additional surgical procedure

5. 5. Rehabilitation

The overall goals of therapy are to arrest the destructive process and to promote healingand reepithelialization of the corneal surface.[72-75]

Topical Steroids

Initial therapy should include intensive topical steroids, consisting of prednisolone

acetate or prednisolone phosphate 1%, hourly in association with topical cycloplegics and

prophylactic antibiotics.[72][73][74][75] If epithelial healing does not occur within 2 to 3days, the frequency of topical steroid application can be increased to every half hour.

Once healing occurs, the frequency can be reduced, and tapered slowly over a period of
http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref65http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref12http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref12http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref66http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref67http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref68http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref69http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref70http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref71http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref71http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref72http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref73http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref74http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref75http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#tophttp://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref65http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref12http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref66http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref67http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref68http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref69http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref70http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref71http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref72http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref73http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref74http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref75


12/14

several months. Such management, especially in unilateral, benign form has met with

good results. Some authors have advocated oral pulse therapy (60 to 100 mg daily of oral

prednisone) when topical therapy is ineffective after 7 to 10 days or in cases wheretopical steroids may be dangerous because of precariously deep ulcer or infiltrate.[9],[68]

Topical tetracycline or medroxyprogesterone may be used for anticollagenolytic

properties of each. A therapeutic soft contact lens or patching of the eye may bebeneficial at this stage. Also, any concomitant eye disease, such as acne rosacea,

meibomeitis, blepharitis, dry eye, or eyelid abnormalities should be addressed.[72]

Conjunctival Resection

If the ulcer progresses despite the steroid regimen, conjunctival resection should be

performed.[72][73][[74][75] Under topical and subconjunctival anaesthesia, this consistsof conjunctival excision to bare sclera extending at least 2 clock hours to either side of

the peripheral ulcer, and approximately 4 mm posterior to the corneoscleral limbus and

parallel to the ulcer.[76] The overhanging lip of ulcerating cornea may also be removed.Postoperatively, a firm pressure dressing should be used.[72],[73] In Mondino's series,

[74] conjunctival resection healed 3 of 4 unilateral ulcers and 3 of 3 bilateral

nonsimultaneous ulcers, but only 2 of 15 bilateral simultaneous ulcers. Tissue adhesive

and a therapeutic soft contact lens may be beneficial. Multiple resections may benecessary. The rationale of this procedure is that the conjunctiva adjacent to the ulcer

contains inflammatory cells that may be producing antibodies against the cornea and

cytokines which amplify the inflammation and recruit additional inflammatory cell.Cryotherapy of limbal conjunctiva has been advocated by some authors and may have a

similar effect.[77],[78] Brown reported that excision of the limbal conjunctiva adjacent to

the progressing ulcer, resulted in healing of the ulcers in 8 of 10 eyes treated; however, itmay be necessary to repeat this procedure three times before a beneficial effect is seen.

[79] On the other hand Stilma studied 38 Mooren's corneal ulcers and found that

conjunctival excision with thermocoagulation gave some relief at the site of the ulcers,

but recurrences at other places occurred in at least 52% of cases.[80]

Kinoshita and colleagues[81] reported success in their series of 20 Mooren's patients

treated with keratoepithelioplasty. In this procedure, donor corneal lenticles are suturedonto the scleral bed after conjunctival excision. These authors postulate that the lenticles

form a biological barrier between host cornea and the reepithelializing conjunctiva and

the immune components it may carry. It should be emphasized, however, that whether ornot the presence of this donor material added a therapeutic effect above and beyond the

resection of tissue in preparation for the graft is unclear. Application of isobutyl

cyanoacrylate, a tissue adhesive, may work in the same way but perhaps more simply andwithout the risk of epithelial rejection, glaucoma secondary to the chronic steroid use

necessitated by keratoepithelioplasty and development of neurotrophic keratitis.[81]

Systemic immunosuppressive chemotherapy
http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref9http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref9http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref68http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref68http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref72http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref72http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref73http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref74http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref75http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref76http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref72http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref73http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref74http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref77http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref78http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref79http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref80http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref81http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref81http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#tophttp://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#tophttp://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref9http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref68http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref72http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref72http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref73http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref74http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref75http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref76http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref72http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref73http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref74http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref77http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref78http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref79http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref80http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref81http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref81


13/14

Those cases of bilateral or progressive Mooren's ulcer that fail the preceding therapeuticattempts will require systemic cytotoxic chemotherapy to bring a halt to the progressive

corneal destruction.[72][73][74][75] The most commonly used agents are

cyclophosphamide (2 mg/kg/day), methotrexate (7.5 to 15 mg once weekly) andazathioprine (2 mg/kg body weight/day). The degree of fall in white blood cell count is

considered as the most reliable indicator of immunosuppression produced by

cyclophosphamide. Foster reported arrest of the inflammatory process and preservationof ocular anatomy and function in 8 of 9 patients with bilateral involvement treated with

immunosuppressives for 6 to 24 months. The ninth patient, who developed perforation,

did not seem to receive an adequate level of immunosuppression as measured by blood

parameters.[82],[83] Mondino has reported that immunosuppressive drugs haltedprogression in 4 of 13 patients with Mooren's ulcer who did not respond to topical

corticosteroids or conjunctival resection. In this series immunosuppressive drugs were

reserved for only the most severe, resistant cases.[84] We disagree with the statement by

Schanzlin[12]that "since the value of immunosuppressive therapy is less clear than othertreatments, it is recommended only in the severest and most resistant cases". On the

contrary, we believe that the evidence for the efficacy of systemic immunosuppressivechemotherapy for progressive bilateral Mooren's ulcer is quite strong, and further believe

that such treatment should be employed sooner rather than later in the care of such

patients, before the corneal destruction has become too extensive to need for surgery.

Agents such as cyclophosphamide may be effective by suppressing B lymphocytes,

which produce autoantibodies and promote immune complex disease.[85] More recently,

oral cyclosporin A (10 mg/kg/day) has been successfully used to treat a case of bilateralMooren's ulcer unresponsive to local therapy with topical corticosteroids, silver nitrate,

and conjunctival resection, as well as systemic immunosuppression with corticosteroids,

cyclophosphamide, and azathioprine.[86] This agent may work by suppression of thehelper T cell population and stimulation of the depressed population of suppressor and

cytotoxic T cells present in patients with Mooren's ulcer.[86],[87] Adverse effects of

these cytotoxic and immunosuppressive medications, such as anaemia, alopecia, nausea,nephrotoxicity and hepatotoxicity, are likely and the administering physician must be

vigilant about their onset. In most instances, systemic immunosuppressive therapy is best

handled by close collaboration between an ophthalmologist and an oncologis.The

potentially serious side effects of systemic immunosuppressive agents have led severalgroups to investigate the efficacy of topical applied cyclosporin A in the treatment

Mooren's ulcer.[88],[89] In Holland's series[90] of a variety of anterior segment

inflammatory diseases; two patients had Mooren's ulcer. The first, with bilateral disease,showed reduced inflammation with minimal progression over 3 years, whereas the

second, with unilateral disease, showed resolution of the ulcer on this medication. In

Zhao and Jin's series[88],15 of 18 eyes with Mooren's ulcer improved, and 11 of thesewere cured with topical cyclosporin A therapy (0.5% solution). In both studies, local or

systemic side effects attributable to topical cyclosporin A were not observed.

Additional Surgical Procedures
http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref72http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref73http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref74http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref75http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref82http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref83http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref84http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref12http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref12http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref85http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref85http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref86http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref86http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref86http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref87http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref87http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref88http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref89http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref90http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref88http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref88http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#tophttp://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref72http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref73http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref74http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref75http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref82http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref83http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref84http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref12http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref85http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref86http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref86http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref87http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref88http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref89http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref90http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref88


14/14

When these management steps fail, additional surgical procedures may be considered.Superficial lamellar keratectomy, has been shown to arrest the inflammatory process and

allow healing.[91] After refining the technique in previous series, Nian and

colleagues[92] found 34 of 40 eyes to be successfully healed after lamellar keratectomy.Four of 6 eyes with recurrent ulcer healed with retransplantation while the other patients

refused further intervention

Some cases may progress to perforation despite management as just detailed. Small

perforations may be treated with application of tissue adhesive and placement of a soft

contact lens to provide comfort and to prevent dislodging of the glue.[72],[73] When a

perforation is too large for tissue adhesive to seal the leak, some type of patch graft willbe necessary. This may range from a small tapered plug of corneal tissue to a penetrating

keratoplasty. In case of larger peripheral perforations, a partial penetrating keratoplasty

may be performed.[73] Rarely conjunctival flaps or even penetrating keratoplasty may be

necessary. It should be emphasized that the prognosis of corneal graft in the setting ofacute inflammation in patients with Mooren's ulcer is very poor.[72],[76]

Rehabilitation

Once the active ulceration has ceased and the remaining cornea has been completely

opacified, it is possible to perform penetrating keratoplasty on these patients, even in theface of a thinned and vascularized cornea.[73],[76] In these instances, a 13 mm tectonic

corneal graft is first sutured in place with interrupted 10-0 nylon or prolene sutures with

the recipient bite extending into the sclera so that the suture will not pull through the thinhost cornea and then a 7.5 or 8.0 mm therapeutic graft is placed.[73],[76]Glaucoma and

cystoid macular oedema have been a problem in these cases, and although the graft

remains clear, the visual result may only be 6/60. Because of the immune system's

remarkable memory, surgical attempts at rehabilitation in Mooren's ulceration should bedone only with concurrent immunosuppression, even when the active disease has been

arrested, or is 'burned out,' because attempts at penetrating keratoplasty often are

associated with recurrence and graft failure.[19],[76],[93] Some authors believe that therisk of recurrence is so great that patients are best served not by any intervention but by

maintaining the current status - i.e., the vision provided by their own thinned, scarred

cornea.[94] It is also believed that patients with treated Mooren's ulcer should beimmunosuppressed prior to cataract surgery or corneal grafting procedure.[61] In absence

of donor corneas Stilma has suggested the usage of a free lamellar scleral autograft to

restore the corneal defect, followed by penetrating keratoplasty later[80]. In his series of38 eyes with corneal ulcer six eyes with a progressive iris prolapse and a flat anterior

chamber were reconstructed with the above technique.
http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref91http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref92http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref72http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref72http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref73http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref73http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref73http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref72http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref76http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref73http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref73http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref76http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref76http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref73http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref73http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref76http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref76http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref19http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref19http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref76http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref76http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref93http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref93http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref94http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref61http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref80http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#tophttp://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref91http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref92http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref72http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref73http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref73http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref72http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref76http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref73http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref76http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref73http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref76http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref19http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref76http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref93http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref94http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref61http://www.ijo.in/article.asp?issn=0301-4738;year=1997;volume=45;issue=1;spage=7;epage=17;aulast=Sangwan#ref80

Mooren Ulcer

Documents

Transcript of Mooren Ulcer